Scleroedema adultorum Buschke

Abstract

Scleredema adultorum of Buschke is a rare disorder of unknown aetiology. It is characterized by diffuse, non-pitting swelling and induration of the skin. Skin biopsies reveal marked thickening of the dermis due to collagenous replacement of the subcutis and deposition of hyaluronic acid between the collagen fibers. The disease classically only affects the skin. In 24 cases an associated monoclonal gammopathy has been detected. A 75-year-old patient had a 19-year history of scleredema adultorum. In addition to a monoclonal gammopathy the patient suffered from involvement of the tongue, pharynx and upper esophagus. Furthermore a polyneuropathy, ocular involvement with restricted eye movements and a sicca syndrome were present. The simultaneous occurrence of cutaneous scleredema with any one of the above mentioned symptoms has been reported before. The wide variety of extracutaneous manifestations of scleredema as found in our patient is amazing and has not been previously described.

Macroglossia and lip edema: A case of paraproteinemia-associated scleredema responsive to intravenous immunoglobulins

“…While extracutaneous manifestations of scleredema have been described, including buccal and parotid involvement, reports of tongue swelling are exceedingly rare. 2 , 3 Ulmer et al 4 published a case of scleredema with tongue, pharyngeal, and esophageal involvement in a 75-year-old male with IgG lambda monoclonal gammopathy and sicca syndrome. Scleredema with tongue swelling can mimic other dermatologic disorders, such as systemic amyloidosis and lipoid proteinosis, which typically present with small lingual papules or ulcerations.…”

Scleroedema adultorum Buschke und exulzerierte Necrobiosis lipoidica bei Diabetes mellitus Typ 1

“…Kennzeichnend sind Ablagerungen von sauren Mukopolysacchariden im dermalen Bindegewebe bei ödematöser Induration der Haut [3,15,16]. Anders als bei der Sklerodermie ist beim Scleroedema in der Histologie das Kollagen nur schwach eosinophil und klinisch eine Organbeteiligung selten [16]. Eine Unterscheidung ist laborchemisch durch das Fehlen von Autoantikörpern (ANAs etc.)…”

Phototherapy and photochemotherapy of sclerosing skin diseases

“…Scleredema adultorum is characterized by rapidly progressive non‐pitting cutanous‐subcutaneous indurations. It is usually a self‐limited disease that regresses in 50% of patients within two years after onset (49). There are reports on the use of bath and cream PUVA in scleredema adultorum (50, 51).…”